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Successful reduced-intensity SCT from unrelated cord blood in three patients with X-linked SCID

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Title: Successful reduced-intensity SCT from unrelated cord blood in three patients with X-linked SCID
Authors: Iguchi, Akihiro Browse this author
Kawamura, Nobuaki Browse this author →KAKEN DB
Kobayashi, Ryoji Browse this author
Takezaki, Shun-ichiro Browse this author
Ohkura, Yuka Browse this author
Inamoto, Jun Browse this author
Ohshima, Junjiro Browse this author
Ichikawa, Mizuho Browse this author
Sato, Tomonobu Browse this author
Kaneda, Makoto Browse this author
Cho, Yuko Browse this author
Yamada, Masafumi Browse this author
Kobayashi, Ichiro Browse this author
Ariga, Tadashi Browse this author →KAKEN DB
Keywords: Stem cell transplantation (SCT)
X-linked severe combined immunodeficiency (XSCID)
reduced-intensity stem cell transplantation (RIST)
cord blood (CB)
Issue Date: Dec-2011
Publisher: Nature Publishing Group
Journal Title: Bone Marrow Transplantation
Volume: 46
Issue: 12
Start Page: 1526
End Page: 1531
Publisher DOI: 10.1038/bmt.2010.338
Abstract: We described three boys with X-linked severe combined immunodeficiency (X-SCID) who were successfully treated by reduced-intensity stem cell transplantation from unrelated cord blood (CB). Mean age at the transplantation was 5.7 months (range, 3-9 months). Pre-transplantation conditioning for all patients consisted of fludarabine (FLU) (30 mg/m2/day) from day -7 to day -2 (total dose 180 mg/m2) and busulfan (BU) 4mg/kg/day from day -3 to day -2 (total dose 8 mg/kg). All of the CB units were serologically matched HLA-A, B, and DR loci. Although two of them had suffered from fungal or bacterial pneumonia before transplantation, there were no other infectious complications during transplantation. All the patients have engrafted and achieved 100% donor chimerism. We also confirmed full donor chimerism of both T and B cells. Only one patients developed acute GVHD grade III, which resolved by increasing the dose of oral corticosteroid. None of the patients have developed chronic GVHD during the follow up for 21 to 77 months. No patients had received intravenous immunoglobulin replacements after the transplantations, or showed delay in psychomotor development. Reduced-intensity conditioning consisting of FLU and BU and transplantation from unrelated CB was effective and safe treatment for patients with X-SCID.
Type: article (author version)
Appears in Collections:北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 井口 晶裕

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